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Aim: There is no consensus on the best prophylaxis for supraventricular tachycardia (SVT) in infancy. We studied the efficacy and safety of sotalol. Method: This retrospective study comprised infants diagnosed with SVT before 1 year of age and treated with sotalol during 2002–2018 in Stockholm, Sweden. The patients' characteristics, comorbidities, sotalol dosages, QT intervals and outcomes were extracted from their medical records. Results: We studied 85 infants (65% boys) with a median age of eight (range 0–288) days at the time of diagnosis, including 78 with re‐entry tachycardia. Sotalol was completely or partially successful in the 67/75 patients who completed the treatment, as well as in four of the seven patients with other tachycardia mechanisms. The 48 infants with postnatal debut had significantly higher success rates than the 27 with foetal debut (96% vs. 78%, p = 0.04). Prolongation of corrected QT (QTc) intervals of ≥450 ms occurred in 16% of the total cohort and two patients with QTc intervals of ≥500 ms had their treatment changed. There were no cases of proarrhythmia after sotalol treatment. Conclusion: Sotalol provided effective and safe prophylaxis for SVT during infancy. QTc prolongation rarely caused treatment discontinuation and there were no cases of proarrhythmia. [ABSTRACT FROM AUTHOR]

An 18-year-old female neutered Domestic Shorthair cat was referred for investigation of seizure-like episodes. Physical and neurological examination were unremarkable, as were systemic arterial blood pressure, cardiac troponin I, complete blood count and biochemistry profile. Diagnostic tests included transthoracic echocardiography which ruled out any structural cardiomyopathy, and electrocardiography which showed an underlying regular wide QRS-complex rhythm with pronounced shortening of the PR interval. Considering the findings, a diagnosis of suspected ventricular pre-excitation secondary to an accessory pathway was made. Further investigations were declined and, as no periods of tachycardia were seen on the five-minute electrocardiogram, no antiarrhythmic treatment was started. [ABSTRACT FROM AUTHOR]

A 72-year-old Brazilian woman presented with a 4-year history of rest tremors of the hands, followed by slowness of movement, and a diagnosis of idiopathic Parkinson's disease. She was started on dopamine agonists with significant improvement. After three years, she complained about slowly progressive dysphagia, dysphonia, quadriparesis, and cramps and fasciculations. A neurological examination disclosed distal-dominant quadriparesis, dysarthria, atrophy and fasciculation of the tongue, global brisk tendon reflexes, fasciculations, bilateral ankle clonus, and moderate spasticity of the lower limbs. She had also palpitations, dyspnea, and one episode of paroxysmal atrial fibrillation. Electrocardiography revealed a short PR interval, a widened QRS complex, and the delta wave, suggestive of Wolff–Parkinson–White syndrome. Brain and spine MR imaging, a cerebrospinal fluid analysis, and general serum lab exams were unremarkable. Needle electromyography disclosed chronic denervation involving cervical, thoracic, lumbosacral, and bulbar levels associated with acute denervation, including positive sharp waves, fasciculations, and fibrillation potentials. This patient fulfilled the diagnostic criteria for amyotrophic lateral sclerosis associated with parkinsonism. A broad next-generation sequencing-based panel disclosed the presence of the novel heterozygous variant c.1247C > T (p.Pro416Leu) in the PRKAG2 gene (NM_016203.4). Clinicians must be aware of the possibility of PRKAG2 variants in complex clinical scenarios associating cardiac arrhythmia, preexcitation syndromes, hypertrophic cardiomyopathy, motor neuron disease, and parkinsonism. [ABSTRACT FROM AUTHOR]

Background Conduction abnormalities are frequently encountered in patients with Ebstein anomaly. The following case describes the safe use of flecainide in an infant with accessory-pathway mediated left ventricular dysfunction in the setting of Ebstein anomaly. Case Summary An infant with an antenatal diagnosis of Ebstein anomaly developed progressive left ventricular dilatation and dysfunction over the first 2 months of life. ECG demonstrated persistent Wolff-Parkinson-White pattern with delta-wave polarity suggesting a right-sided septal accessory pathway. In the absence of SVT, accessory-pathway mediated dyssynchrony was suspected as the cause for left ventricular dilatation and dysfunction. He was commenced on flecainide which successfully blocked antegrade conduction via the accessory pathway resulting in a reduction in left ventricular volume and improvement in left ventricular systolic function. He remains asymptomatic at 12 months of age. Discussion There is a known association between Ebstein anomaly and Wolff-Parkinson-White pattern. Right-sided septal accessory pathways can cause cardiomyopathy secondary to dyssynchronous left ventricular contraction. In patients who are unsuitable for accessory pathway ablation, flecainide can be used to block antegrade conduction via the accessory pathway resulting in improved left ventricular function, which was successful on this occasion. [ABSTRACT FROM AUTHOR]

Identifying and treating pediatric arrhythmias is essential for pediatric anesthesiologists. Pediatric patients can present with narrow or wide complex tachycardias, though the former is more common. Patients with inherited channelopathies or cardiomyopathies are at increased risk. Since most pediatric patients present for anesthesia without a baseline electrocardiogram, the first identification of an arrhythmia may occur under general anesthesia. Supraventricular tachycardia, the most common pediatric tachyarrhythmia, represents a broad category of predominately narrow complex tachycardias. Stimulating events including intubation, vascular guidewire manipulation, and surgical stimulation can trigger episodes. Valsalva maneuvers are unreliable as treatment, making adenosine or other intravenous antiarrhythmics the preferred acute therapy. Reentrant tachycardias are the most common supraventricular tachycardia in pediatric patients, including atrioventricular reciprocating tachycardia (due to a distinct accessory pathway) and atrioventricular nodal reentrant tachycardia (due to an accessory pathway within the atrioventricular node). Patients with ventricular preexcitation, often referred to as Wolff‐Parkinson‐White syndrome, have a wide QRS with short PR interval, indicating antegrade conduction through the accessory pathway. These patients are at risk for sudden death if atrial fibrillation degenerates into ventricular fibrillation over a high‐risk accessory pathway. Automatic tachycardias, such as atrial tachycardia and junctional ectopic tachycardia, are causes of supraventricular tachycardia in pediatric patients, the latter most typically noted after cardiac surgery. Patients with inherited arrhythmia syndromes, such as congenital long QT syndrome, are at risk of developing ventricular arrhythmias such as polymorphic ventricular tachycardia (Torsades de Pointes) which can be exacerbated by QT prolonging medications. Patients with catecholaminergic polymorphic ventricular tachycardia are at particular risk for developing bidirectional ventricular tachycardia or ventricular fibrillation during exogenous or endogenous catecholamine surges. Non‐selective beta blockers are first line for most forms of long QT syndrome as well as catecholaminergic polymorphic ventricular tachycardia. Anesthesiologists should review the impact of medications on the QT interval and transmural dispersion of repolarization, to limit increasing the risk of Torsades de Pointes in patients with long QT syndrome. This review explores the key anesthetic considerations for these arrhythmias. [ABSTRACT FROM AUTHOR]

Background: Published guidelines provide recommendations for risk stratification in pediatric Wolff-Parkinson-White (WPW). There are no data on provider concordance with these guidelines. We hypothesized that significant practice variation exists between pediatric cardiologists (PC) and electrophysiologists (EP). Method: The records of all patients, age 8 to 21 years, with a new ECG diagnosis of WPW between 1/1/2013 and 12/31/2018, from a single center, were retrospectively reviewed. Subjects were categorized on the basis of symptoms and resting ECG findings as one of the following: asymptomatic intermittent WPW, asymptomatic persistent WPW, or symptomatic WPW. The performance and results of diagnostic testing, including Holter monitor, event monitor, exercise stress test (EST), and electrophysiology study (EPS), were recorded. The primary outcome was concordance with published guidelines. A secondary outcome was documentation of a discussion of sudden cardiac death (SCD) risk. Results: 615 patient encounters were analyzed in 231 patients with newly diagnosed WPW pattern on ECG (56% male; mean age at diagnosis 13.9 ± 2.5 years). EP were observed to have a significantly higher rate of guideline concordance than PC (95% vs. 71%, p < 0.001). There was significant practice variation between PC and EP in the documentation of a discussion of SCD risk: 96% in EP vs. 39% in PC (p < 0.001). Conclusion: Significant practice variation exists in the non-invasive and invasive risk stratification of pediatric WPW, with lower concordance to published guidelines amongst PC, when compared to EP. This report highlights the need to promote awareness of current WPW guidelines in the pediatric cardiology community at large. [ABSTRACT FROM AUTHOR]

A 72-year-old Brazilian woman presented with a 4-year history of rest tremors of the hands, followed by slowness of movement, and a diagnosis of idiopathic Parkinson’s disease. She was started on dopamine agonists with significant improvement. After three years, she complained about slowly progressive dysphagia, dysphonia, quadriparesis, and cramps and fasciculations. A neurological examination disclosed distal-dominant quadriparesis, dysarthria, atrophy and fasciculation of the tongue, global brisk tendon reflexes, fasciculations, bilateral ankle clonus, and moderate spasticity of the lower limbs. She had also palpitations, dyspnea, and one episode of paroxysmal atrial fibrillation. Electrocardiography revealed a short PR interval, a widened QRS complex, and the delta wave, suggestive of Wolff–Parkinson–White syndrome. Brain and spine MR imaging, a cerebrospinal fluid analysis, and general serum lab exams were unremarkable. Needle electromyography disclosed chronic denervation involving cervical, thoracic, lumbosacral, and bulbar levels associated with acute denervation, including positive sharp waves, fasciculations, and fibrillation potentials. This patient fulfilled the diagnostic criteria for amyotrophic lateral sclerosis associated with parkinsonism. A broad next-generation sequencing-based panel disclosed the presence of the novel heterozygous variant c.1247C > T (p.Pro416Leu) in the PRKAG2 gene (NM_016203.4). Clinicians must be aware of the possibility of PRKAG2 variants in complex clinical scenarios associating cardiac arrhythmia, preexcitation syndromes, hypertrophic cardiomyopathy, motor neuron disease, and parkinsonism.

The non-invasive localization of accessory pathway (AP) in patients with Wolff-Parkinson-White (WPW) syndrome is typically performed upon physicians’ diagnoses based on observing their electrocardiogram (ECG) signals, which are not always the same. Therefore, a high-accuracy automatic method can help minimize this gap regarding AP localization. This study was to develop a novel semi-automatic localization of AP in patients with WPW syndrome, using features selected from the cross-recurrence plot (CRP) of consecutive precordial leads on ECG. The study participants comprised of 31 patients with WPW syndrome (aged 8-69, with the mean age of 31.19±14.69, 32.3% female), receiving successful ablation therapy during the first session. The features extracted from the CRP, including laminarity (LAM), trapping time (TT), determinism (DET), and mean length of diagonal line (L) were then analyzed. The feature reduction, The classification and the cross-validation (CV) methods were sequential forward selection (SFS), the k-nearest neighbors (KNN) and the leave-one-out (LOO) respectively. The proposed method could differentiate the right and left APs in the patients with WPW syndrome with the accuracy value of 87% (sensitivity: 93.33%, specificity: 81.25%). These results were achieved by the LAM and L features from the CRP of (V1, V2) and (V3, V4), respectively.

Fetal autoimmune atrioventricular block (AVB) is a rare but potentially lifethreatening condition. It results from the passage of maternal anti-SSA/Ro or Anti SSB/La auto-antibodies into the fetal circulation, leading to inflammation and fibrosis of the AV node and often to irreversible damage. Besides AVB, these antibodies can also cause cardiomyopathies, but there is no evidence linking them to tachyarrhythmias. We present the case of a patient with significant risk factors for fetal AVB: a prior history of hydrops fetalis, high anti-SSA/Ro antibody levels and hypothyroidism. In this case, the use of dexamethasone and intravenous immunoglobulin may have contributed to reversing the first degree atrioventricular block detected at 19 weeks of gestation. Additionally, at 21 weeks, the fetus developed a tachyarrhythmia that needed treatment with flecainide. Soon after the birth, the newborn underwent ECG Holter and Wolff-Parkinson-White Syndrome (WPWS) was diagnosed. To our knowledge, the coexistence of fetal AVB and WPWS has never been described. [ABSTRACT FROM AUTHOR]

Introduction: Pregnant women with pre-excitation syndrome are more likely to develop supraventricular tachycardia (SVT) during pregnancy and delivery, leading to an increased risk of adverse events. Method: This was a retrospective study of 309 pregnancies in 280 women (29 women had two pregnancies in this series) with pre-excitation syndrome who delivered at West China Second University Hospital from June 2011 to October 2021. All the 309 pregnant women with pre-excitation syndrome were divided into SVT and non-SVT groups to analyze the cardiac and obstetric complications. Results: Among the included pregnant women in the past 10 years, the prevalence of pre-excitation syndrome was 0.24% (309/127725). There were 309 cases with pre-excitation syndrome in all hospitalized pregnant women. Among them, 62 (20.1%, 62/309) had a history of SVT. In the 62 cases with SVT during pregnancy, 22 (35.5%) cases had a history of SVT. Gestational diabetes mellitus was associated with SVT during pregnancy. The cesarean section rate was 88.7% in the SVT group, which was significantly higher than that in the non-SVT group (64.8%) (P < 0.001). Cases with SVT during pregnancy had more cardiac and obstetric complications. Four fetal deaths were recorded in the SVT group. Additionally, 29 women experienced two pregnancies during the study period, among whom, five received radiofrequency ablation after the first delivery and obtained better outcomes in the second pregnancy. Conclusion: The adverse outcomes such as cardiac complications, maternal and fetal complications (PROM, prematurity, SGA, fetal distress, etc.) in pregnant women with pre-excitation syndrome were closely related to SVT, with possible risk factors including history of SVT before pregnancy, cardiac function, heart organic abnormalities, and gestational diabetes mellitus. [ABSTRACT FROM AUTHOR]

Background: T‐wave memory (TWM) is a rare cause of T‐wave inversion (TWI). Alterations in ventricular activation due to abnormal depolarization may cause repolarization abnormalities on the ECG, even if myocardial conduction returns to normal. These repolarization changes are defined as TWM. In our study, we aimed to determine the frequency of TWM development and the predictors affecting it in the pediatric population who underwent accessory pathway (AP) ablation due to Wolff‐Parkinson‐White (WPW) syndrome. Methods: The data of patients with manifest AP who underwent electrophysiological studies and ablation between 2015 and 2021 were retrospectively analyzed. The study included 180 patients who were under 21 years of age and had at least one year of follow‐up after ablation. Patients with structural heart disease, intermittent WPWs, recurrent ablation, other arrhythmia substrates, and those with less than one‐year follow‐up were excluded from the study. The ECG data of the patients before the procedure, in the first 24 h after the procedure, three months, and in the first year were recorded. The standard ablation technique was used in all patients. Results: Postprocedure TWM was observed in 116 (64.4%) patients. Ninety‐three patients (51.7%) had a right‐sided AP, and 87 patients (48.3%) had a left‐sided AP. The presence of posteroseptal AP was found to be significantly higher in the group that developed TWM. Of these patients, 107 (93.1%) patients showed improvement at the end of the first year. Preprocedural absolute QRS‐T angle, postprocedural PR interval, and right posteroseptal pathway location were identified as predictors of TWM. Conclusion: The development of TWM is particularly associated with the right‐sided pathway location, especially the right posteroseptal pathway location. The predictors of TWM are the preprocedural QRS‐T angle, the postprocedural PR interval, and the presence of the right posteroseptal AP. [ABSTRACT FROM AUTHOR]

Ablation of accessory pathways (APs) is the cornerstone for treatment of patients with Wolff-Parkinson-White syndrome and manifestation of atrioventricular reentrant tachycardia. Pulsed field ablation (PFA) is a new type of nonthermal energy source delivered to the underlying tissue via the ablation catheter and used for ablation of arrhythmic substrates. The purpose of this study was to determine the efficiency and long-term outcome of ablation of APs of different localizations using a focal pulsed electrical field. Electrophysiological study was performed in patients with indication for AP ablation. An ablation catheter was used to map the position of AP insertion. Pulsed electric field was delivered through a standard ablation catheter. In left-sided APs, the first ablation attempt was within the coronary sinus (CS). Patient follow-up was scheduled 1–3 months after the ablation. Additional check-up was performed after 6 and 12 months. Fourteen 14 patients (3 pediatric) were treated. Termination of AP conduction was achieved in all procedures. The cohort consisted of 3 right free wall, 3 posteroseptal, and 8 left-sided APs. Ablation through CS was successfully used in 7 of 8 patients with left-sided APs. No complications were reported. Median follow-up was 5.5 months. Conduction recurrence through AP was documented in 1 patient. Focal PFA for AP shows promising results in terms of efficacy and safety. A high rate of successful termination of left-sided APs by ablation within CS may represent a new standard approach. The safety and efficacy profile of PFA seems to be transferable to the pediatric population. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 69-year-old female who underwent an electrophysiological study due to recurrent supraventricular tachycardia (SVT). It highlighted the occurrence of a wide complex tachycardia (WCT) and explored the mechanism behind its transformation following a premature ventricular complex (PVC). Treatment discussed involved radiofrequency ablation targeting the base of the triangle of Koch, which successfully eliminated SVT inducibility and potentially the NFV pathway.

Wolff-Parkinson-White (WPW) syndrome is a rare cardiac condition arising from abnormal embryologic development of the annulus fibrosus in combination with the cardiac conduction system. The abnormality results in the development of accessory pathways and preexcitation changes which can provoke episodes of tachyarrhythmias. The most common presentation of WPW syndrome is supraventricular tachycardia. Beyond customary abortive therapy, chronic management strategies vary based upon timing and clinical severity of the initial disease presentation. Prompt diagnosis and rate control have a dramatic impact on the outcomes of morbidity and mortality. The purpose of this article is to present a case study of a preterm infant who manifested with WPW syndrome. Additionally, the article will explore the pathophysiology of WPW syndrome and the timing and presentation of common clinical manifestations of the disease, along with current diagnostic and treatment strategies to achieve optimal patient outcomes in the neonatal population. [ABSTRACT FROM AUTHOR]

Introduction: Williams syndrome (WS) cases have been reported to have with 25–100 times greater increased risk of sudden cardiac death (SCD). SCD has been reported in cases without any evidence of structural cardiovascular anomalies. Wolff–Parkinson–White (WPW) syndrome is characterized by short PR interval and delta wave. Ventricular preexcitations can develop paroxysmal reentrant tachycardia through Kent bundle or less frequent atrial fibrillation and in some cases with accessory pathway effective refractory period (APERP) under 250 ms considered as risky and may lead to SCD. WS associated with WPW has not been reported before. Case Report: An 11‐year‐old male who had been followed up with WS was referred to pediatric cardiology outpatient clinic with the complaint of palpitation. Electrocardiographic examination showed short PR interval and delta wave in the ECG consistent with WPW. He underwent electrophysiological study (EPS). Basic measurements were performed, and APERP was found at 280 ms cycle atrial pacing. RF energy was delivered using a 4 mm tip nonirrigated radiofrequency (RF) ablation catheter where the best ventriculoatrial (VA) signals were received and the AP was abolished within few seconds. Discussion and Conclusions: Although, WPW cases are usually asymptomatic or related to SVT, the risk of SCD should not be ignored. Thus, all patients with WPW deserve an EPS for assessing the AP conduction properties. Due to the increased risk of SCD in patients with WS compared to general population, in the presence of concomitant WPW, these patients should be evaluated with EPS even if they do not have symptoms. [ABSTRACT FROM AUTHOR]

Introduction: Precise electrocardiographic localization of accessory pathways (AP) can be challenging. Seminal AP localization studies were limited by complexity of algorithms and sample size. We aimed to create a nonalgorithmic method for AP localization based on color‐coded maps of AP distribution generated by a web‐based application. Methods: APs were categorized into 19 regions/types based on invasive electrophysiologic mapping. Preexcited QRS complexes were categorized into 6 types based on polarity and notch/slur. For each QRS type in each lead the distribution of APs was visualized on a gradient map. The principle of common set was used to combine the single lead maps to create the distribution map for AP with any combination of QRS types in several leads. For the validation phase, a separate cohort of APs was obtained. Results: A total of 800 patients with overt APs were studied. The application used the exploratory data set of 553 consecutive APs and the corresponding QRS complexes to generate AP localization maps for any possible combination of QRS types in 12 leads. Optimized approach (on average 3 steps) for evaluation of preexcited electrcardiogram was developed. The area of maximum probability of AP localization was pinpointed by providing the QRS type for the subsequent leads. The exploratory data set was validated with the separate cohort of APs (n = 256); p =.23 for difference in AP distribution. Conclusions: In the largest data set of APs to‐date, a novel probabilistic and semi‐automatic approach to electrocardiographic localization of APs was highly predictive for anatomic localization. [ABSTRACT FROM AUTHOR]

The article present case study of a 52-year-old woman who had been previously diagnosed with type-A Wolff-Parkinson-White (WPW) syndrome. Topics include the diagnostic assessment of a narrow QRS tachycardia; differential diagnosis between orthodromic reciprocating tachycardia (ORT) and atrioventricular nodal reentrant tachycardia (AVNRT); and the complexities in distinguishing a posteroseptal atrioventricular pathway from a rare nodoventricular pathway.

The European Society of Cardiology (ESC) was established in 1949 by a group of European participants at the Inter-American Congress of Cardiology. It was officially founded in 1950 and held its first congress in 1952. In 1980, the ESC established the European Heart Journal (EHJ), which has become a successful academic and economic venture. The ESC has grown from a club of friends to an institution, with various associations, councils, and working groups. It has also established the European Heart House and the European Heart Agency. The ESC has defined a 5-year strategy and aims to be inclusive, transparent, and diverse. It focuses on providing trusted knowledge, embracing digital health and artificial intelligence, and addressing ethical and regulatory challenges. The ESC also prioritizes advocacy, sustainable finances, and reducing the global burden of cardiovascular disease. [Extracted from the article]

For left-sided radiofrequency catheter ablation (LCA) in pediatrics, significant practice variability exists regarding anticoagulation and discharge practices. Given the lack of data in pediatric patients, the risks and benefits of these practices are not well defined. The purpose of this study was to evaluate the safety of same-day discharge and use of aspirin (ASA) in pediatric patients following LCA. We performed a retrospective cohort study of pediatric patients who underwent LCA from 2010 to 2020 at our institution. Discharge timing and ASA usage were based on operator preference. The primary outcome was incidence of postablation anticoagulation complications reported within 1 month of the procedure. Three hundred seventy-six patients underwent LCA and met inclusion criteria. Median [25th, 75th percentiles] age was 13.9 [10.5, 16.2] years; 18 (4.7%) had a history of structural heart disease. The most common substrates for ablation were Wolff-Parkinson-White syndrome (183 patients [48.7%]), concealed accessory pathway (159 patients [42.3%]), and ectopic atrial tachycardia (10 patients [2.7%]). Three hundred thirty-eight patients (89.9%) were discharged on the day of LCA. Seventy-six patients (20.2%) were prescribed ASA at discharge. Of those who underwent follow-up (273 patients [72.6%]), 7 (2.7%) reported an anticoagulation complication (5 with hematoma, 2 with headache). One of these patients was prescribed ASA; none required readmission. There was no correlation between anticoagulation complications and same-day discharge or with ASA usage. Given the rare incidence of anticoagulation complications in pediatric patients undergoing LCAs, same-day discharge from the electrophysiology laboratory without anticoagulation should be considered. [ABSTRACT FROM AUTHOR]

Copyright of Revista Argentina de Terapia Intensiva is the property of Sociedad Argentina de Terapia Intensiva (SATI) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Wolff-Parkinson-White (WPW) syndrome is defined by specific electrocardiogram (ECG) changes resulting in ventricular pre-excitation (the so-called WPW pattern), related to the presence of an accessory pathway (AP), combined with recurrent tachyarrhythmias. WPW syndrome is characterized by different supraventricular tachyarrhythmias (SVT), including atrioventricular re-entry tachycardia (AVRT) and atrial fibrillation (AF) with rapid ventricular response, with AVRT being the most common arrhythmia associated with WPW, and AF occurring in up to 50% of patients with WPW. Several mechanisms might be responsible for AF development in the WPW syndrome, and a proper electrocardiographic interpretation is of pivotal importance since misdiagnosing pre-excited AF could lead to the administration of incorrect treatment, potentially inducing ventricular fibrillation (VF). Great awareness of pre-excited AF’s common ECG characteristics as well as associated causes and its treatment is needed to increase diagnostic performance and improve patients’ outcomes. In the present review, starting from a paradigmatic case, we discuss the characteristics of pre-excited AF in the emergency department and its management, focusing on the most common ECG abnormalities, pharmacological and invasive treatment of this rhythm disorder. [ABSTRACT FROM AUTHOR]

The article presents the discussion on case study of 17-year-old healthy male with chest pain and intermittent episodes of shortness of breath. Topics include risk stratification and possible catheter ablation of AP due to the small but well-characterized risk of sudden death associated with asymptomatic pre-excitation; and ongenitally corrected transposition of the great arteries, and hypertrophic cardiomyopathy appears to confer a higher risk of multiple APs.

Copyright of Herzschrittmachertherapie und Elektrophysiologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Herzschrittmachertherapie und Elektrophysiologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Herzschrittmachertherapie und Elektrophysiologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

BackgroundDespite the high success rate of radiofrequency catheter ablation (RFCA) in Wolff-Parkinson-White Syndrome (WPW), localizing the successful ablation site can be challenging and may require multiple radiofrequency (RF) applications.ObjectiveThis study aims to evaluate the efficacy of a novel workflow for the automatic and precise identification of accessory pathway ablation site, named Delta Wave Automatic Mapping.MethodsPatients undergoing a first procedure for RF ablation of a manifest accessory pathway were included. Electro-Anatomical Mapping (EAM) was performed with the CARTO 3 system (Biosense Webster, Johnson & Johnson Medical S.p.a., Irvine, CA) leveraging auto-acquisition algorithms already present in the CARTO 3 software. Mapping and ablation were performed with an irrigated tip catheter with contact force sensor. Procedure success was defined as loss of pathway function after ablation. The number of RF applications required and time to effect were measured for each patient. Recurrences were evaluated during follow-up visits. Additionally, at the end of each procedure, historical predictors of ablation success were measured offline to evaluate their relationship with the successful ablation site found with the novel workflow.ResultsA total of 50 patients were analysed (62% APs right and 38% APs left). All 50 APs were successfully eliminated in each procedure with a median Time-to-effect (TTE) of 2.0 (IQR 1.2–3.5) seconds. No AP recurrences during a median follow-up of 10 (IQR 6–13) months were recorded. Offline analysis of successful ablation site revealed a pre-ablation delta/ventricular interval of ≤−10 msec in 52% of the patients and in 100% of the patients the signal related to the Kent bundle was identified.ConclusionsThe novel workflow efficiently localizes APs without requiring manual annotations. Historical endocardial parameters predicting success were measured offline for each case and they corresponded to the ablation target automatically annotated by the proposed workflow. This novel mapping workflow holds promise in enhancing the efficacy of RFCA in the presence of manifest APs.

A 12-lead electrocardiogram of a pediatric patient with Wolff–Parkinson–White syndrome was consistent with the anteroseptal accessory pathway. The patient had three ablation procedures because of the recurrences of the arrhythmia. In our case, successful cryoablation was performed in the non-coronary cusp of the aortic root.

ABSTRACT Objective This study aimed to provide a long-term follow-up of PRKAG2 syndrome and describe the new phenotypic aspects of the condition. PRKAG2 syndrome is a rare autosomal-dominant glycogen storage disease characterized by cardiac hypertrophy, ventricular pre-excitation, and conduction system disease. Fatal arrhythmias occur frequently. Methods A family cohort of 66 participants was recruited. Clinical and genetic analyses were performed. Results Median age of 36.97±17.28 years, with 69.9% being men. Nineteen subjects carried the deleterious variant p.K290I of the PRKAG2 gene. This group experienced many malignant events, including eight pacemaker implants, three sudden cardiac deaths, five aborted cardiac arrests, four strokes, four premature neonatal deaths, two spontaneous abortions, five forceps deliveries, and 12 cesarean procedures. Extracardiac involvement, such as in neurocognitive and psychiatric disorders, has been observed only in carriers of mutations. Palpitations, Syncope, atrial fibrillation, atrial flutter, sinus pauses, and bradycardia were strongly and significantly associated with major or severe adverse events (sudden cardiac death, aborted cardiac arrest, pacemaker use, stroke, and congestive heart failure). Early diagnosis and intervention through antiarrhythmic drugs, anticoagulation, pacemaker implantation, radiofrequency catheter ablation, and cesarean section surgery improved the symptoms and survival rates. Mutations carriers were advised to avoid pregnancy. Conclusion This study identified that the p.K291I_PRKAG2 mutation is associated with poor prognosis, highlighting the need for early intervention. Further research may uncover the potential connections between intellectual disability, miscarriage, and neonatal death in individuals with this syndrome.

Fetal autoimmune atrioventricular block (AVB) is a rare but potentially life-threatening condition. It results from the passage of maternal anti-SSA/Ro or Anti SSB/La auto-antibodies into the fetal circulation, leading to inflammation and fibrosis of the AV node and often to irreversible damage. Besides AVB, these antibodies can also cause cardiomyopathies, but there is no evidence linking them to tachyarrhythmias. We present the case of a patient with significant risk factors for fetal AVB: a prior history of hydrops fetalis, high anti-SSA/Ro antibody levels and hypothyroidism. In this case, the use of dexamethasone and intravenous immunoglobulin may have contributed to reversing the first-degree atrioventricular block detected at 19 weeks of gestation. Additionally, at 21 weeks, the fetus developed a tachyarrhythmia that needed treatment with flecainide. Soon after the birth, the newborn underwent ECG Holter and Wolff-Parkinson-White Syndrome (WPWS) was diagnosed. To our knowledge, the coexistence of fetal AVB and WPWS has never been described.

This article is an editorial that discusses the use of pharmacological therapy in patients with arrhythmias. It provides information on the prevalence and incidence of different types of arrhythmias, such as supraventricular tachycardia and atrial fibrillation, and their associated risk factors. The article emphasizes the importance of early detection and maintenance of sinus rhythm in patients with atrial fibrillation. It also discusses the use of catheter ablation and additional pharmacological therapy in managing arrhythmias. The article concludes by mentioning the importance of anticoagulation therapy and the potential benefits of direct-acting oral anticoagulants in certain patient populations. The given document is a list of references for various research articles related to heart disease, stroke, and atrial fibrillation. These articles cover topics such as the epidemiology of atrial fibrillation, predictors of specific types of atrial fibrillation, the impact of ablation on mortality, remote monitoring systems for implantable cardiac defibrillators, and the use of artificial intelligence in the treatment of atrial fibrillation. [Extracted from the article]

The ECG of a patient during sinus rhythm shows preexcited QRS pattern, with rS pattern in lead V1, transition in lead V2, and positive inferior leads. Following the stepwise algorithms, the location of accessory pathway (AP) was identified at anteroseptal region. However, the precordial transition in lead V2 indicates mid-septal or posteroseptal AP. The mismatch suggested multiple APs and 5 APs were identified by electrophysiologic study. This case highlights the importance of detailed analysis of ECG in order to achieve adequate ablation. [ABSTRACT FROM AUTHOR]

This article discusses the importance of evaluating for cardiac rhabdomyomas in patients with infantile hemangiomas (IH) and tuberous sclerosis complex (TSC) before initiating treatment with propranolol. The case study presented describes a patient with IH and hypopigmented macules who was found to have multiple cardiac rhabdomyomas in the context of TSC. The article emphasizes the need for dermatologists to be familiar with TSC diagnostic criteria in order to make appropriate referrals for further evaluation. Cardiology screening is necessary in patients with TSC to rule out rhabdomyomas with associated arrhythmias or obstructed blood flow before starting treatment. [Extracted from the article]

Long QT syndrome (LQT) and WPW syndrome are causes of sudden cardiac death (SCD) in the young, and their association has been rarely reported. A 26-year-old woman presented with recurrent syncope. Her ECG showed a short PR interval, wide QRS (150 ms) due to a delta wave, and QT prolongation (QT 580 ms, QTc 648 ms). ECG monitoring documented recurrent salvos of a self-terminating wide QRS tachycardia, generally slightly polymorphic, sometimes with "torsade des pointes" (TdP) appearance, which were linked to the syncopal/presyncope episodes. Electrophysiologic monitoring diagnosed a right para-hisian accessory pathway with a very short ERP (240 ms baseline, <200 ms after isoproterenol). The pathway was ablated successfully. Despite QRS narrowing (80 ms), QT prolongation persisted after ablation (QT 620 ms, QTc 654 ms), with short runs of TdP, despite beta-blocker treatment, which was increased to the maximal dosage. A dual-chamber implantable cardioverter defibrillator (ICD) was implanted. To our knowledge, this is the first case report of an association between LQT and WPW syndrome in which both conditions are associated with an increased risk of SCD. [ABSTRACT FROM AUTHOR]

Wolf–Parkinson–White (WPW) syndrome is a disorder characterized by the presence of at least one accessory pathway (AP) that can predispose people to atrial/ventricular tachyarrhythmias and even sudden cardiac death. It is the second most common cause of paroxysmal supraventricular tachycardia in most parts of the world, affecting about 0.1–0.3% of the general population. Most patients with WPW syndrome have normal anatomy, but it may be associated with concomitant congenital heart disease or systemic diseases. Although many individuals are asymptomatic, during supraventricular arrhythmia episodes, they may experience severe symptoms, including syncope or even sudden cardiac death (mainly due to pre-excited atrial fibrillation over rapidly conducting AP). In addition to arrhythmia-related symptoms, for some specific locations of the APs with overt anterograde conduction, there might be a reduction in exercise capacity mediated by a reduction in LV systolic performance due to anomalous LV depolarization. Although it is typically diagnosed through electrocardiography (ECG), additional tests are necessary for risk assessment. Management of WPW syndrome may be quite challenging and can vary from only acknowledging the presence of the accessory pathway to pharmacological treatment or radiofrequency ablation. Early diagnosis, risk assessment, and appropriate treatment are critical steps in the management of WPW syndrome, aiming to improve the quality of life and reduce the risk of life-threatening arrhythmias. [ABSTRACT FROM AUTHOR]

Limited data are available concerning supraventricular tachycardia (SVT) recurrence. Hence, this study aimed to determine the incidence, outcome, and factors associated with SVT recurrence. This retrospective, observational, population-based study was conducted among children with SVT from 2006 to 2020. The primary outcome measure was SVT recurrence. Kaplan Meier analysis was used to estimate SVT-free at 1, 5, and 10 years after diagnosis. Cox regression analysis was used to identify independent factors associated with recurrence. There were 156 patients with SVT with a median age at diagnosis of 1.9 years (Interquartile range [IQR] 11 days to 8.7 years) and follow-up for a median of 3.5 years (IQR 1.7 to 6.1 years). 35 patients (22%) had recurrent SVT at a median age of 7.8 years (IQR 4.4 to 12 years). Infants with Wolff-Parkinson-White Syndrome (WPW) had the highest recurrence (11/16, 68%), with 33% SVT-free at 5 years follow-up. The lowest recurrence rate was observed in neonatal diagnosis (2/54, 3.7%) with 98% SVT-free at 5 years follow-up. The independent factors associated with the recurrence of SVT were the diagnosis of WPW with an adjusted hazard ratio (aHR) of 5.2 (95% CI 2.4–11.2), age of more than 1 year at diagnosis (aHR 3.7 95% CI 1.4–9.7), and combine with or need second-line therapy (aHR 4.0 95% CI 1.5–10.7). One in five children with SVT experienced a recurrence, which is more likely for those with WPW, multiple maintenance therapy, and older age at first presentation. Whereas neonates with non-WPW may benefit from shorter maintenance therapy. [ABSTRACT FROM AUTHOR]

Aims Controversy remains as to whether the exercise stress test (EST) is sufficient for risk evaluation in patients with pre-excitation. This study aims to clarify the usefulness of EST in risk stratification in both asymptomatic and symptomatic patients presenting with pre-excitation. Methods and results This prospective study includes consecutive asymptomatic and symptomatic patients with pre-excitation referred for risk assessment. All participants performed an incremental EST (bicycle) prior to an electrophysiology study (EPS). Primary data from the EST included loss of pre-excitation during exercise, and primary data from the EPS included the measurement of accessory pathway effective refractory period (APERP), shortest pre-excited RR interval (SPERRI), and inducible arrhythmia with the use of a beta-adrenergic receptor agonist if deemed necessary. One hundred and sixty-four patients (59 asymptomatic, 105 symptomatic) completed an EST and EPS. Forty-five patients (27%) demonstrated low-risk findings on EST, of which 19 were asymptomatic and 26 were symptomatic. Six patients with low-risk EST findings had SPERRI/APERP ≤ 250 ms at EPS, and two of them were asymptomatic. The sensitivity, specificity, positive predictive value, negative predictive value (NPV), and accuracy of low-risk EST for excluding patients with SPERRI/APERP ≤ 250 ms were 40, 91, 87, 51, and 60%, respectively. The number of patients with inducible arrhythmia at EPS was similar in the asymptomatic (36, 69%) and symptomatic (73, 61%) groups. Conclusion Sudden loss of pre-excitation during EST has a low NPV in excluding high-risk APs. The EPS with the use of isoproterenol should be considered to accurately assess the risk of patients with pre-excitation regardless of symptoms (ClinicalTrials.gov Identifier: NCT03301935). [ABSTRACT FROM AUTHOR]

Background: Wolff-Parkinson-White syndrome is a rare but well-known disease that predisposes a person to cardiac arrhythmias. But sometimes this syndrome is accompanied by heart failure in the affected person. Several causes have been proposed for this complication including recurrent or incessant tachyarrhythmias that are frequently found in symptomatic (especially children) Wolf-Parkinson-White syndrome patients causing heart dysfunction and dilation and dilated cardiomyopathy, and also pre-excitation-related dyssynchrony leading to progressive ventricular remodeling and dilation. In this report, a patient is introduced who has improved his heart failure by radio-frequency ablation of free wall type of this syndrome. Case Presentation: A 35-year-old man who had a history of Wolff-Parkinson-White syndrome from 8 years ago and suffered from dilatation and reduced left ventricular ejection fraction in recent years was presented with a pre-excited atrial fibrillation attack at the emergency department. The initial surface ECG showed positive delta wave in all precordial leads and negative QRS complexes in interior leads with QRS duration of about 200 ms. He had undergone electrophysiology study and ablation at the left postero-lateral accessory pathway. After ablation of accessory pathway within the months (from November 2022 until June 2023), left ventricular function was gradually improved and the symptoms of the patient's shortness of breath were also decreased. Conclusion: The existence of heart failure in patients with Wolff-Parkinson-White syndrome can be due to various reasons including the presence of an accessory pathway and the dyssynchrony of intraventricular contraction which is caused by premature excitation of the connected part of the left ventricle by accessory atrioventricular pathway. Although in order to rule out the possibility of the incidental association of the accessory pathway with primary dilated cardiomyopathy and to investigate the segmental dyskinesia, it is necessary to perform diagnostic measures such as echocardiography and cardiac computerized tomography and magnetic resonance imaging, ablation of such accessory pathway not only controls arrhythmic attacks but also leads to the improvement of the left ventricular systolic function even in a middle age patient. [ABSTRACT FROM AUTHOR]

There is a rich history of surgery for cardiac arrhythmias, spanning from atrial fibrillation and Wolff–Parkinson–White syndrome to inappropriate sinus tachycardia and ventricular tachycardia. This review describes the history of these operations, their evolution over time, and the current state of practice. We devote considerable time to the discussion of atrial fibrillation, the most common cardiac arrhythmia addressed by surgeons. We discuss ablation of atrial fibrillation as a stand-alone operation and as a concomitant operation performed at the time of cardiac surgery. We also discuss the emergence of newer procedures to address atrial fibrillation in the past decade, such as the convergent procedure and totally thoracoscopic ablation, and their outcomes relative to historic approaches such as the Cox maze procedure. [ABSTRACT FROM AUTHOR]

Introduction: Acquired Wolff–Parkinson–White (WPW) syndrome can occur after congenital heart disease (CHD) surgery. Methods and Results: A 27‐year‐old male with Ebstein's anomaly and manifest WPW syndrome received catheter ablation twice. The first electrophysiology study (EPS) induced orthodromic atrioventricular reentrant tachycardia and successfully eliminated the posteroseptal accessory pathway (AP). Six months after the Cone procedure, the patient suffered from palpitation. The second EPS was performed and abolished the right lateral AP. Conclusion: The appearance of a new AP after the reconstruction of CHD is a rare finding and should raise suspicion of an acquired AP connection. [ABSTRACT FROM AUTHOR]

During catheter ablation (CA) of tachycardia, conventional point‐by‐point mapping may be hindered due to hemodynamic instability. We performed open‐window mapping (CARTO, Biosense Webster, USA) in a patient who developed hemodynamic instability during orthodromic atrioventricular reciprocating tachycardia (AVRT) and unwarranted induction of AVRT during attempts to map accessory pathway (AP) with ventricular pacing. With over 11,000 points acquired rapidly, the system accurately identified AP at the mitral valve (MV) annulus where the application of single radiofrequency (RF) lesion promptly eliminated AP's conduction. Our case illustrates the utility of open‐window mapping for the ablation of AVRT. [ABSTRACT FROM AUTHOR]

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This article discusses a case of a 16-year-old boy with left ventricular hypertrophy (LVH) and preexcitation on an electrocardiogram (ECG). The boy presented with symptoms of shortness of breath and swelling of the feet, and further examination revealed LVH and severe impairment of the left ventricular ejection fraction. The presence of preexcitation in a background of LVH may indicate underlying conditions such as Danon disease, PRKAG2 mutation cardiomyopathy, Fabry disease, Pompe disease, or mitochondrial disorders. In this case, the patient was diagnosed with Danon disease, a rare X-linked disorder characterized by cardiomyopathy, skeletal myopathy, and intellectual disability. The article emphasizes the importance of careful interpretation of ECG findings and the need for clinical correlation and genetic testing to determine the underlying cause of heart failure. [Extracted from the article]

Delta wave morphology during sinus rhythm on 12 lead ECG usually allows for a relatively precise prediction of the anatomic location of an accessory pathway (AP) provided that the rhythm is fully pre-excited. However, the pre-excitation pattern during sinus rhythm usually offers little information about sudden cardiac death (SCD) risk in adult patients with asymptomatic Wolf-Parkinson-White (WPW). A 42-year-old policeman was referred for an asymptomatic WPW pattern with an unusual delta wave. Initial mapping showed that atrio-ventricular (A-V) fusion on the lateral mitral annulus and radiofrequency (RF) ablation induced a shift in the pre-excitation pattern. Re-mapping identified A-V fusion on the left postero-septal area. Another RF application eliminated pre-excitation. Re-mapping identified A-V fusion on the postero-septal aspect of the mitral annulus and another RF application almost instantaneously induced an A-V split and loss of pre-excitation. Post-ablation testing proved normal anterograde A-V conduction without pre-excitation and no ventriculo-atrial conduction. Serial ECGs proved the persistent loss of pre-excitation at one-month of follow-up. This case highlights the diagnostic challenges associated with atypical pre-excitation patterns and the importance of a thorough evaluation in asymptomatic individuals. Patients with atypical pre-excitation may harbor multiple accessory pathways (MAPs) and should be carefully assessed to mitigate the risk of SCD. [ABSTRACT FROM AUTHOR]

Introduction: Herein, we present a rare case of the successful ablation of an accessory pathway (AP) involving the Marshall Bundle (MB) and coronary sinus musculature (CSM) in a 40‐year‐old man with Wolff−Parkinson−White syndrome. Methods and Results: An orthodromic reciprocating tachycardia (ORT) was inducible with the earliest atrial activation site located at the posterolateral mitral annulus. The local conduction and the cycle length of ORT was prolonged by peri‐mitral ablation; however, it failed to block the AP. The atrial insertion of the AP was identified by remapping during ORT at the left atrial ridge, which was away from the mitral annulus, where ablation was successful. Together with the electrophysiological findings in CSM potentials, we conclude that the epicardial MB‐CSM connection functioned as the AP in this patient. Conclusion: The novel variant form of AP comprised of MB and CSM should be noted. The atrial insertion of the MB may be the target of catheter ablation. [ABSTRACT FROM AUTHOR]